Prion Biology and Diseases, 2nd Ed. (2004, Volume 41)

Cover Page

This volume is a new edition of the most authoritative book on Prion Biology, first published in 1999 and edited by the Nobel Prize-winning founder of the field. This expanded edition has been completely updated, and includes chapters on therapeutics, and diagnostic methods and approaches.


"Prion Biology and Diseases is by far the most authoritative introduction and summary of the complex data that form the basis of our understanding of the molecular basis of this esoteric group of diseases. While the human forms of the diseases are rare, their economic impact, particularly in the United Kingdom, has been profound. Moreover, their occurrence is now threatening the normal operation of blood-transfusion services worldwide. Hence the need for such an authoritative book. It is the best comprehensive overview of this field and is an ideal introduction for anyone wishing to launch a career in prionology."


"All in all, this book is a good read. All the necessaries are there but the style is light and accessible. I would recommend this book to anybody, whether they work in the field and want a concise summary of the state of prion research, or are only now entering the fascinating world of the prion."

-Trends in Neurosciences

"This book is a testament to the tremendous contributions of Prusiner and his collaborators in the field of prion research."

-New England Journal of Medicine

"Prusiner has brought together an impressive amount of scientific data dealing with prions (a word he coined). This book is a must for anyone interested in the prion field. For those not familiar with the field, the book provides ample introduction. The book is an invaluable reference source combining clinical, epidemiological, genetic, and biochemical data. An exhaustive amount of material is presented dealing with everything from how to isolate prions to strategies for analyzing PrP structure."


Table of Contents

Preface/Front Matter
Stanley B. Prusiner
Excerpt       PDF     (i-xiii)
1 An Introduction to Prion Biology and Diseases
Stanley B. Prusiner
Excerpt       PDF     (1-87)
2 Development of the Prion Concept
Stanley B. Prusiner
Excerpt       PDF     (89-142)
3 Bioassays of Prions
Stanley B. Prusiner, Jiri Safar, Stephen J. Dearmond
Excerpt       PDF     (143-186)
4 Transmission and Replication of Prions
Stanley B. Prusiner, Michael R. Scott, Stephen J. Dearmond, George Carlson
Excerpt       PDF     (187-242)
5 Structural Studies of Prion Proteins
Cédric Govaerts, Holger Wille, Stanley B. Prusiner, Fred E. Cohen
Excerpt       PDF     (243-282)
6 Doppel, a New PrP-like Mammalian Protein
David Westaway, Leroy E. Hood, Stanley B. Prusiner
Excerpt       PDF     (283-304)
7 Prions of Yeast and Filamentous Fungi: [URE3], [PSI+], [PIN+], and [Het-s]
Reed B. Wickner, Susan W. Liebman, Sven J. Saupe
Excerpt       PDF     (305-372)
8 Knockouts, Knockins, Transgenics, and Transplants in Prion Research
E. Flechsig, J.C. Manson, R. Barron, A. Aguzzi, C. Weissmann
Excerpt       PDF     (373-434)
9 Transgenetic Investigations of the Species Barrier and Prion Strains
Michael Scott, David Peretz, Rosalind M. Ridley, Harry F. Baker, Stephen J. Dearmond, Stanley B. Prusiner
Excerpt       PDF     (435-482)
10 Cell Biology of Prions
David A. Harris, Peter J. Peters, Albert Taraboulos, Vishwanath Lingappa, Stephen J. Dearmond, Stanley B. Prusiner
Excerpt       PDF     (483-544)
11 Scrapie, Chronic Wasting Disease, and Transmissible Mink Encephalopathy
Stanley B. Prusiner, Elizabeth Williams, Jean-Louis Laplanche, Morikazu Shinagawa
Excerpt       PDF     (545-594)
12 Bovine Spongiform Encephalopathy and Related Diseases
Gerald A. H. Wells, John W. Wilesmith
Excerpt       PDF     (595-628)
13 Infectious and Sporadic Prion Diseases
Robert G. Will, Michael P. Alpers, Dominique Dormont, Lawrence B. Schonberger
Excerpt       PDF     (629-671)
14 Inherited Prion Diseases
Qingzhong Kong, Witold K. Surewicz, Robert B. Petersen, Wenquan Zou, Shu G. Chen, Pierluigi Gambetti, Piero Parchi, Sabina Capellari, Lev Goldfarb, Pasquale Montagna, Elio Lugaresi, Pedro Piccardo, Bernardino Ghetti
Excerpt       PDF     (673-775)
15 Neuropathology of Prion Diseases
Stephen J. Dearmond, James W. Ironside, Essia Bouzamondo-Bernstein, David Peretz, Jan R. Fraser
Excerpt       PDF     (777-856)
16 Some Strategies and Methods for the Study of Prions
Stanley B. Prusiner, Giuseppe Legname, Stephen J. Dearmond, Fred E. Cohen, Jiri Safar, Detlev Riesner, Kiyotoshi Kaneko
Excerpt       PDF     (857-920)
17 Biosafety Issues in Prion Diseases
Henry Baron, Jiri Safar, Darlene Groth, Stephen J. Dearmond, Stanley B. Prusiner
Excerpt       PDF     (921-960)
18 Therapeutic Approaches to Prion Diseases
Stanley B. Prusiner, Barnaby C. H. May, Fred E. Cohen
Excerpt       PDF     (961-1014)
Excerpt       PDF     (1015-1022)
Appendix Prion Protein (PrP) and Doppel (Dpl) Sequences
Kurt Giles, Stanley B. Prusiner
Excerpt       PDF     (1023-1032)
Excerpt       PDF     (1033-1050)